A cerebral dural arteriovenous fistula (DAVF) is an acquired abnormal arterial-to-venous connection within the leaves of the intracranial dura with a wide range of clinical presentations and natural history. The Cognard classification correlates venous drainage patterns with neurological course, identifying 5 DAVF types with increasing rates of symptomatic presentation. A spinal DAVF occurs when a radicular artery makes a direct anomalous shunt with a radicular vein within the dural leaflets of the nerve root sleeve. A cervical DAVF is a rare entity, as most spinal DAVFs present as thoracolumbar lesions with myelopathy. In this paper the authors present 2 patients presenting initially with brainstem dysfunction rather than myelopathy secondary to craniocervical DAVF. The literature is then reviewed for similar rare aggressive DAVFs at the craniocervical junction presenting with brainstem symptomatology.